Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only widely effective therapies for these patients. We report a case of PNH disorder which presented with abdominal pain and mild tenderness. Abdominal X ray and sonography revealed intra-peritonealfree fluid and air. At laparotomy, an ischemic segment of jejunum with stricture and perforationwith mesenteric venous thrombosis were found. The patient also had a history of hepatic vein thrombosis two years before.

کلیدواژه ها:

Paroxysmal Nocturnal Hemoglobinuria ، Intestinal Perforation ، Thrombosis ، Abdominal Pain